| Long-term functional outcomes in children with Currarino syndrome. | |
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MedLine Citation:
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PMID: 20473613 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: The aim of the study was to review the degree to which the long-term outcome and ongoing morbidity in Currarino syndrome (CS) has been established. METHODS: Analysis of previously published reports that have included long-term outcome data in CS and review of five additional patients with CS. RESULTS: Overall, long-term outcomes of children born with CS are not well described. Malignancy has been reported in six children of approximately 300 CS patients: four children with malignancy had a recurrence after primary excision. Malignancy has also occurred in four adults. Ongoing morbidity related to constipation, faecal incontinence, neurogenic bladder, urinary incontinence and presacral abscess, and more rarely meningitis, brain metastases, developmental delay and unusual gait. Almost certainly, previous reports have under-estimated the true incidence of these problems, given the methodology and focus of these series. CONCLUSIONS: There is paucity of information on the long-term outcomes in CS. Few authors have focused on ongoing symptoms, such that we speculate the true incidence of long-term urinary and bowel dysfunction may have been under-estimated in CS. Greater emphasis on the functional assessment of these systems during childhood may help predict the long-term outcome in CS. The most severe cases are diagnosed during infancy and childhood, and these are also the ones who are more likely to have ongoing long-term morbidity. |
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Authors:
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Atsushi Yoshida; Kiki Maoate; Russell Blakelock; Stephen Robertson; Spencer Beasley |
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Publication Detail:
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Type: Case Reports; Journal Article; Review Date: 2010-05-15 |
Journal Detail:
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Title: Pediatric surgery international Volume: 26 ISSN: 1437-9813 ISO Abbreviation: Pediatr. Surg. Int. Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-06-18 Completed Date: 2010-09-17 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8609169 Medline TA: Pediatr Surg Int Country: Germany |
Other Details:
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Languages: eng Pagination: 677-81 Citation Subset: IM |
Affiliation:
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Department of Paediatric Surgery, Christchurch School of Medicine and Health Sciences, Christchurch Hospital, Private Bag 4710, Christchurch, New Zealand. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Abnormalities, Multiple
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genetics,
surgery* Anus, Imperforate / genetics, surgery* Child Constipation / etiology Constriction, Pathologic / etiology, surgery Dermoid Cyst / pathology, surgery Female Follow-Up Studies Humans Infant, Newborn Intestinal Obstruction / etiology Male Prognosis Rectal Diseases / genetics, surgery* Retrospective Studies Sacrum / abnormalities* Syndrome Teratoma / pathology, surgery |
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